Clinical analysis of 13 cases of maxillofacial neuroendocrine carcinoma

LIANG Yi-ran; ZHAO Rui; WEN Wei-sheng; LYU Yan; WANG Yi-zhu; GUO Bin

doi:10.19538/j.kq.2018.02.011

Abstract：Objective To investigate the clinical characteristics and prognosis of maxillofacial neuroendocrine carcinoma(NEC).Methods A retrospective study was performed,including 13 patients with maxillofacial neuroendocrine carcinoma which was diagnosed by clinical pathology in General Hospital of PLA between January 1997 and January 2016.The general clinical information,treatment,pathological examination and clinical prognosis were analyzed.Results Among the 13 patients,4 were male (30.8％)and 9 were female (69.2％)witha mean age of 60.4±4.8 years old.The course of the disease was aggressive.Two cases (15.4％) were primary cancer without metastasis;9 cases (69.2％)metastasized to adjacent organ and regional lymph node;2 cases (15.4％) metastasized to distant organ (ovary and lung).Maxillofacial neuroendocrine carcinoma location in 13 cases was:5 in parotid gland,3 in upper/lower jaw,1 in tongue,1 in face,1 in submandibular area and 1 in neck.The hematoxylin-eosin (HE)pathological staining showed the histological type was small cell type.Tumor immunohistochemical analysis found that 7 cases (53.8％) were chromogranin A (CgA) positive,11 cases(84.6％)were synapsin(Syn)positive and 10 cases (76.9％) were neuron-specific enolase(NSE)positive.The cases with Ki-67 proliferation index≥＞50％ accounted for 69.2％,and Ki-67 proliferation index ＜ 50％ 30.8％.The 5-year survival rate was 15.4％.Thirteen patients were undergone surgery.Adjuvant radiotherapy and chemotherapy were performed according to the patient's condition except two patients who refused adjuvant treatment.5 cases suffered tumor recurrence and 2 cases had internal organ metastasis.Only 2 cases survived at the end of follow-up investigation.Conclusion Maxillofacial NEC shows no specific clinical manifestation.NEC is prone to develop lymph node metastasis and adjacent organs metastasis.Surgical operation is the primary treatment so far.Postoperative adjuvant radiotherapy and chemotherapy may be beneficial to patients,but the overall prognosis is poor.